Abstract
We encountered a very rare case of bile duct carcinoma associated with congenital biliary dilatation (CBD) in a 16-year-old female who was admitted to our hospital because of right upper abdominal pain and vomiting. Abdominal computed tomography demonstrated a cystic dilatation of the common bile duct measuring 7 cm in diameter and two enhanced tumors 4 cm in diameter located in the inferior bile duct and middle bile duct. Magnetic resonance cholangiopancreatography clearly demonstrated a cystic dilatation of the extrahepatic bile duct (Todani’s CBD classification: type 4-A). Endoscopic retrograde cholangiopancreatography also revealed two tumors. Biopsy results of one of the tumors confirmed adenocarcinoma. Excision of the perihilar bile duct and subtotal stomach-preserving pancreaticoduodenectomy with dissection of the major lymph nodes were performed. A postoperative histopathologic examination revealed a well-differentiated tubular adenocarcinoma, which remained within the mucosal layer, and no lymph node metastasis was found. The postoperative course was uneventful, and the patient was discharged 10 days after surgery and has remained disease-free for 21 months.
Highlights
It is well known that congenital biliary dilatation (CBD) has a significant association with bile duct carcinoma and that CBD merges with pancreaticobiliary maljunction (PBM) in all cases [1]
PBM is a congenital anomaly defined as a junction of the pancreatic and biliary ducts located outside of the duodenal wall that usually forms a markedly long common channel [2, 3]
We report a rare case of a 16-year-old female with early bile duct carcinoma combined with PBM and CBD
Summary
It is well known that congenital biliary dilatation (CBD) has a significant association with bile duct carcinoma and that CBD merges with pancreaticobiliary maljunction (PBM) in all cases [1]. We report a rare case of a 16-year-old female with early bile duct carcinoma combined with PBM and CBD. Our patient was autistic, her condition did not play any role in her present illness Magnetic resonance cholangiopancreatography (Fig. 3) clearly demonstrated a cystic dilatation of the extrahepatic bile duct (Todani’s CBD classification: type 4-A) [1]. Endoscopic retrograde cholangiopancreatography (Fig. 4) demonstrated a cystic dilatation of the bile duct as well as the presence of two tumors. The resected specimen demonstrated tumors in the inferior and middle bile ducts (Fig. 5). The postoperative course was uneventful, and the patient was discharged 10 days after surgery and has remained disease-free for 21 months
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